Chilly No More!! (For now at least...)

Hey there!  I'm so excited to report that we have had at least 3 days of BEAUTIFUL, sunny weather!  As my friend put it, I am solar-powered.  :)  Some sun on my face and being outside is just what the doctor ordered!  I know rain is on the way tomorrow, but I am hoping that spring has truly sprung.  But you never know with Memphis in March - March is when we have had ice storms and tons of snow and all that.  As Elle says, "GO-way wain!" (Go away, rain!)

So Milla has done pretty good with the scheduled valium - maybe a touch more tired but not much - and her tremors and/or seizures are better!  

Last night, she woke up crying around 10:30pm and cried for about an hour and at one point she got out the word, 'help.'  Totally heart-breaking not being able to fix it.  Her breathing was fast and labored for a bit but recovered after awhile.  I feel like these times of discomfort and sadness, although few and relatively far-between, are becoming more frequent.  And I so wish she could tell me what is wrong and what she needs.  Please pray that we are able to comfort her and that she will not feel fear or sadness or pain.

Elle had a not-so-great week but the sunshine and outside air did her good over the weekend.  During the week, her rages (crazy fits) were 24/7 (or close to it).  I felt like an abuse-victim of verbal and physical assaults at the hand of my pre-schooler.  It was so discouraging and stressful and it definitely had caught up with me by the end of the week.  I felt utterly exhausted, beat-up and so worn down emotionally.  But fortunately, she calmed down over the weekend, although yesterday, she had a TON of 'checking-out.'  Again, not sure if it is seizure activity or lack of brain connectivity, but they were split-second episodes that were literally every 5-10 seconds pretty much all day.  We hate seeing her like that - goes without saying.  Please pray that we will know how best to treat her and help her. (Oh and on a super-encouraging note, Elle is still taking her meds well!!!!!)

I truly do feel like we are all a bit refreshed from the sunshine and playing at the park this weekend.  Ready to face-down another week!

Thank you for EVERYthing, dear friends and family!!!

This has given me hope and comfort this week:

"Keep the broken-hearted sure,

Clinging to Thy cross, our cure."

We cling to Jesus.

Dana

Ann Carlyle and Elle running around at the park :)

Chilly Monday Update

Good Monday morning!  It's chilly and gloomy here today so I'm glad to be done with some of the morning's errands and can hole up with Milla until I have to go pick up Elle from her little school.  

Milla has homebound school right now, so I have a minute to send an update.  And speaking of, I just have to say what a blessing her teacher has been.  Homebound school is a program through the city school system to provide education for kids who have special needs and who can't attend a regular school.  Milla's teacher is phenomenal. She comes twice a week for an hour on Mondays and Thursdays.  She is so dedicated to the kids she teaches and she cares for Milla so much.  These sessions help maintain Milla's skills as much as possible - and she absolutely loves it - so it's a happy part of the week for me too.  :)

So it's been a tough week, honestly.  (Although yesterday both Milla and Elle had a great afternoon - probably because Nana and Granddad came to visit!)  We thought that Milla's tremors were getting worse, but it turns out much of that activity was actually seizures.  So we are changing up her meds again...playing with the dosages of her current meds and adding scheduled valium.  I've been pretty worried about the valium but so far she's done okay with it.  We are upping the dose (of valium) and weaning back another med today, so time will tell how her body will handle that.

Her seizures have pretty much been under control for awhile now, so it was a bit of a blow to hear that her meds weren't working as well anymore.  It just chips away a little bit more of my heart.  I know things are always progressing and that Milla's and Elle's brains are broken but some days it is harder to deal with than others.

And Elle continues to fall and trip and lose her balance and we are definitely seeing drop seizures now.  (The rather violent ones that throw her body around.)  The seizures are all of 1 second long and fortunately, she is only having around a handful a day, but that number will continue to go up and it's just ugh.  We feel that she isn't having enough to warrant more medicine yet - we are going to wait it out for now.

Oh but one awesome thing I've been excited to tell y'all about is that Elle has been doing GREAT at taking her meds the past week/week and a half!!!  Thank y'all for praying and praise the Lord!!!  I can't tell you how wonderful this is and how much this affects my day (as she takes meds 3x a day) and my state of mind.  It's sooooo great.  Pray that this continues!

Ann Carlyle is doing well - I've said this before, but I'll say it again - her school (Christ Methodist Day School) is just plain awesome.  Her teacher is amazing.  She had a President's Day Program Friday with a couple speaking parts and she nailed it.  :)  We are so, so thankful for the opportunity to have her there.  And her eating continues to improve so thank you again for the meals!

Elle, Ann Carlyle and I have been dealing with colds this past week but we have all turned a corner and are on the up and up.  Amazingly and thankfully, Milla never got it.  I saw on the news the other day that the flu is apparently alive and well, so please pray for everyone's health, but specifically Milla, as she is the most medically fragile.  (Although, that girl is probably the strongest one of all 5 of us!!)

As always, I am ever grateful for you, our village!

Much love and coziness today!

Dana

We had a super warm weekend and Milla had an incredible burst of energy yesterday - check out this picture from the park with Uncle Kent!

Feelin' the Love

Just a quick update for the week - 

I had 2 of my oldest and dearest friends come visit me last weekend.  And it was so wonderful.  My soul got filled back up.  :)

And then Elle decided to flood the kitchen Sunday (we have hardwood floors in there) and my soul might have emptied out just a bit again.  Sigh.

Moving on...

We had a good appointment with the girls' neurologist last Friday and we decided to change a couple of things.

For Elle, we are trying to remove any medicine she doesn't absolutely need because she is resisting taking her medications.  A lot.  So we were able to take away the one for her appetite, since it doesn't seem to be working anyway.  And we tried to take away the melatonin (for helping her fall asleep) but that was a complete disaster so we put her right back on that.

But all in all, she's actually been better about taking her meds this week.  Pray that this continues.  Because bottomline, if she refuses to take her meds, our only other option is a G-tube.  And we are certainly not ready for that yet.

Elle's had a couple of good days and a few bad days this week with her seizure-like activity.  But because most of it is probably not seizures, but brain degeneration, we aren't changing any of her seizure medications at this time.  Although, she has had more of the drop seizures, so we might be headed in that direction.

Milla has been about the same with lots of tremors and some bad nights crying off and on.  But we increased one of her seizure meds, hoping it will help with the tremors and decreased another seizure med to counteract any side effects like tiredness, etc.  

The good news is that her tremors are better and she's not been crying the last couple of nights!  The bad news is that I've noticed she's a bit more tired (during the day) lately.  Hopefully this will all balance out though after a few more days.  We will see.

It's always science mixed with art with all these medications.  You want to find that balance between giving the girls quality of life, but not making them zombies.  And that fine balance is constantly changing as their conditions change.  Please pray we are balancing well!

Oh and a quick note about Ann Carlyle - she is liking the new food that y'all are bringing!  The new menu plan is working!  Already she is feeling more in control of her dinner because she is able to anticipate what these things will taste like.  And that has led to dinner-time being less of a battlefield.  Hallelujah.  So thank all you meal-bringers again for sticking to these recipes...you are contributing greatly to our quality of life!

That's about it, I think!

Happy Thursday to all and Happy Valentines Day in advance!  I'm certainly feeling the love - so thank you!! :)

Dana

Nothing like a morning nap with my Milla.  Soul - filled back up.

Orlando!

Hello all!  Just wanted to check in with everyone...

The big news this past week is that we took a 5-day trip to Orlando!  Some very kind and amazing people (to put it mildly!) flew us down to Orlando to stay at a beautiful resort down there complete with water slides, multiple pools, a lazy river, wonderful places to eat and even a Starbucks on-site!  And while we were there, we hit up SeaWorld and Discovery Cove. 

We decided to keep things somewhat simple so we didn't do DisneyWorld but we did some pretty cool things like swimming with dolphins and that.was.awesome.  Elle was technically too young to get to swim with them but she was able to touch one and get in the water for a quick group picture.  

The trip was definitely exhausting but it was worth it.  Amazing memories that we will always treasure.

Yesterday, we got back in the swing of things...back to school, work, schedules, etc.  Re-entry into the real world wasn't as bad as it usually is.  Yay!

We have an appointment with the girls' neurologist tomorrow - not sure if we will have med changes or not, but it's possible.

Elle is still losing a bit of function every day.  (She is actually doing better today.)  But her balance and coordination continue to decline and we are seeing some tremors, especially when she is trying to use her fine-motor skills.  Also, she is having more and more seizure or seizure-like activity.  She may be having drop-seizures now.  (These are the ones that we had such a hard time controlling with Milla...it causes an 'accelerated' drop or fall of the head, trunk or whole body.)  If it is seizure activity, then a medication adjustment or addition may help, but if it is just her brain not making connections due to degeneration, there's not much we can do.  That is a hard reality to swallow.  

Milla is also getting weaker.  We seem to be in a visibly declining state with both of them these days.  Too, she's been having some nights that she is up a lot whimpering.  Please pray that we can figure out the cause of this and/or something that will fix it.  We don't know if she is dreaming or is in pain or just needs a bit of comforting.  She's having a harder time saying the few words she can still say as she is weaker and her tongue tremors are significant as are the tremors with her whole body.  Her hands pretty much stay in a flexed position (wrist and hand at a 90 degree angle) when she is awake and many times one hand is clenched in a fist.  She has a hard time releasing her grip on things.  And she is starting to choke a bit more while eating.  We see her out-of-it more and more.  

All these things make us sad and fearful - goes without saying - so please pray that, while we keep a sense of the reality of our situation, we would be able to just take things one day at a time and not live in fear of the future.  That we would not forget about Ann Carlyle's needs and that, as we seek out those amazing belly-laughs from all our girls, we would soak up the snuggles and be at peace with each day.  That we would have the strength to show up and love and care for each other.  That we would remember that God already has our days numbered and that doubt, guilt and all-encompassing fear is not of Him.  That only by Him and because of Him are we able to live.

Oh and I want to also say thank you to all that are bringing us dinners and thank you so much for going along with our changes to the meal-plan.  We need some consistency for Ann Carlyle so that she will start eating better and with more variety - and at least with her, I can stop being a short-order cook.  (And please pray that she would comply more and more - without the battle.)

Thank y'all for sticking with us.  Having you by our side is life-changing.

Dana 

(Pictures below!)

PS - Thank you for buying the book!!!  The response has been amazing!!!  I'm so glad that it has touched so many lives.  :)

For those who need the link to it... 

http://www.amazon.com/gp/product/1519421532?keywords=voyage%20to%20the%20star%20kingdom&qid=1454621355&ref_=sr_1_1&s=books&sr=1-1

On our way and super excited!!

Our fearless and amazing helpers - Dana's sister, Dawn and our friend, Reg

Always gotta have our sister-almost-twins shot. (PS - I might have the most amazing sister in the world...)

Ann Carlyle lost her other upper tooth when we got to Orlando Her toothless lisp is adorable.  :)

Milla and the manta ray

Ann Carlyle and Elle watching dolphins

Mommy and Milla soaking up the sun

Silly sister time

Discovery Cove

Ann Carlyle swimming with the dolphin!

Frazer and Milla with 'our' dolphin - they are so velvety soft

Ann Carlyle and the unimpressed dolphin

Look how big that dolphin is!

A quick update from Dad

A quick update from dad.

Elle's seizures have increased and generally, she is doing worse, which is to be expected with a degenerative disease, I guess.  Her appetite is not great, her attitude is not great in that she gets frustrated easily and has a very short temper.  She seems to be close to adding morning nap as her sleep during the night is less.  She rarely seeps past 515, and typically she is up before that.  On the bright side, it is a fun challenge to come home from work and try to get her laughing and playing each day.

Milla is generally weaker and her energy level is down.  She seems to have some minor blood circulation issues which don't really need to be dealt with, just another thing we see.  Her appetite has held up pretty good, but her tremors have increased.  Also, we are noticing her vision seems to be worse.  Or, she has trouble controlling her eye muscles to focus on what she wants to look at, its hard to tell the difference.  Her spirits are good and she still loves to giggle and be silly.  Its fun coming home and getting her to laugh!

I was in the kitchen last week and it hit me (more than it usually does) how labor intensive our world is with the girls, which does not let up and is overwhelming at times.  Its also been pretty overwhelming this month to deal with the degenerative nature of Battens.  It never really settles, rarely are the girls ever doing better and we face a long road ahead, but with all that, we have come this far thanks only to God's love and grace He pours out on our family and we plug along!  We thank all of you for pouring out your hearts, time, gifts, prayers, love, meals, flowers and all you have done for us and continue doing for us.  Whether you know it or not, you are being used by God to strengthen and sustain us!  He provides us the strength to make it through each day and there isn't much more for us to ask for right now.  I typically seem to cope and plug along much better when I focus only on my next step.  Very short sighted vision seems to be very effective and we look for our manna each day.  When I provide updates to friends and family or here on email/blog, it forces me step back and assess and opens my eyes to the degenerative nature, but it also opens my eyes to all that God has done to bring us this far and what He does for us each day and week.

Thank you for loving us and caring for us.  I am sorry that I cannot write as well as Dana, but we wanted to be diligent about getting more regular updates out.  I  promise Dana will send the next updates as she is much much better at these than I am, but please know that we are grateful for the love and support that you continue to put out on us!

Fraze

What?? So Soon??!

Hello again!  Just 1 week and here I am.  And at the risk of exposing my self-pride - I am awesome.

So quick update...

Elle had a rough start to the week.  Last Tuesday, she screamed all.day.long.  It was rough.  She just wasn't feeling good and falling a lot and acting like she had just gotten off the tilt-a-whirl.  She's also been getting up around 4/4:30am most days.  So that is contributing to her feeling yucky as well.  She's done a bit better over the weekend although yesterday, she tagged her eye and has a nice cut above and below her left eye.  It's swollen and colorful but it's okay (in that we didn't have to take her in to get stitches or anything).

Milla is also doing better this weekend than she was last week.  Much of the week, she was out-of-it and although she had some great bursts of energy, we can see her overall strength and alertness continue to diminish slowly.  Also, her tremors were more exaggerated.  We are expecting to get some wrist/hand braces in the next week or 2 to help with hand-positioning and possibly her tremors.  She seems a bit more alert this weekend although she has had poor sleep off and on this week.  

We are seeing some extra neediness and attitude from Ann Carlyle, which is hard because, for better or worse, we expect her to be more independent and obedient.  

So while it was a rough week, things improved this weekend - probably because Nana and Granddad (my parents) were here!  :)  

We are hoping to get in for a checkup soon with the girls' neurologist  - possibly this week.  

Thank you to all who have bought the book (Voyage to the Star Kingdom) and for all your kind words!  

And that about sums it up!

Much love!

Dana

2016

So, Happy Thanksgiving, Merry Christmas and Happy New Year!

I know I've been quiet for awhile - part of that was trying to take things off my plate to slow down (if possible) and enjoy the holidays, to remember why we celebrate Christmas, to just be with my family.  November and December were a bit of a dark time for me.  My heart was heavy and I felt like a robot, just going through the motions.  I had emotionally shut down.  I've had these times here and there over the past year - times that have always come and gone - although this one seemed to last a bit longer.  Thankfully, I feel like I've been pulled out of that particular mire and feel like my footing is indeed secure again.

Honestly, we have struggled a bit with the whole "happy new year" thing.  Because we know there are more hard times ahead...probably even harder than we've known.  At first, I felt angry and bitter about it.  What is there to be happy about??  What is there to look forward to??  Which also made me reflect on the past year with the same heartache.  But then.  Then my heart felt a change - begrudgingly at first.  (Make no mistake, this was God changing my heart and reminding me.)  All the people and meals and gifts and time and service and prayers and encouragement that we have experienced!  The love that we might have never known had we not been in this situation.  The faithfulness of God and seeing Him show up again and again.

This is a perfect segue into telling you about the book. :)   The book that 2 sweet friends (the sister and cousin of one of my oldest and dearest friends, Laura Capitell Balzer) have written and illustrated, who were inspired by our girls and the community that surrounds us and the things that God is up to...the reason we have hope...the hope, not in things seen, but in things unseen, the hope in things to come.

The book is called Voyage to the Star Kingdom, by Anne Riley, illustrated by Amy Grimes.  And it's amazing.  It has been in the works for awhile now - many of you have already heard about it.  You can get it on Amazon tomorrow!!!  Here's the link...

http://www.amazon.com/Voyage-Star-Kingdom-Anne-Riley/dp/1519421532/ref=asap_bc?ie=UTF8

See more about Anne on her website http://annerileybooks.com and Amy at http://www.storypaintings.net

As for us...how are we doing?  I've been getting that question a whole lot lately, especially since it's been almost 2 months of radio-silence.  

2 things:

#1 - I am going to try (really really really hard) to update the blog more frequently.  My goal is weekly.  I'm shooting myself in the foot not writing about our comings and goings since the whole reason for this blog was to keep everyone in the know about how we are doing.  (That sentence had way too many 'sayings' in it.  I apologize.)  Anyway, my goal is to update weekly...a short blurb about how the week has been, new prayer requests, etc.

#2 - So how are we doing?  Well, it's complicated, right?  Standard answer, we are hanging in there.  

Milla is trending downward slowly.  Mostly, we see she is losing strength and is having a hard time getting her eyes to find what she's looking for (for example, if I say something to her, she takes awhile to find where I am and then has trouble finding my eyes).  She is still eating well and was actually drinking really well until a couple of days ago.  We tried to wean one of her seizure meds a few of weeks ago, but immediately, her tremors went through the roof, so we had to go right back up to the original dose.  But she remains a happy kid and sweet as can be and for this, we are so very thankful.  

Elle is also trending downward slowly.  (We are thankful, though, that it's not quickly right now.)  Her seizures are mostly small but are, along with her general brain degeneration, really affecting her balance.  She also is still not wanting to eat much.  Too, I worry a lot about her not wanting to take her meds.  But our go-to lately has been getting Ann Carlyle to give Elle her meds.  It's a win-win!  Ann Carlyle loves that Elle wants her to do it...Ann Carlyle may sigh and roll her eyes at times, pretending it's such a hassle, but she loves being given such an awesome job.  :)  (She is 6 ½ yrs old going on 13 for sure.)

And speaking of, Ann Carlyle is doing good.  She asks some tough questions some times and she has been connecting more of the dots, even realizing that when Milla and Elle die, she won't have any sisters anymore.  I can't tell you how much my heart is utterly broken, hearing her come to this realization, but God is truly providing comfort to her little heart as we sit in sadness with each other, giving lots of hugs and reminding myself as much as I remind her how much God loves us.  But all in all, she is still super silly and a little feisty and is a fashionista and loves school and loves being with friends and loves legos.

Christmas was really good - Frazer and I really felt like we had a wonderful balance of seeing most of the family (on both sides) but also having some great time with just the 5 of us.  My heart was trained on some simple but staggering truths of the birth of Jesus - young Mary and all that was required of her...the shepherds in the dead of night, all of the sudden seeing and hearing a bazillion angels singing, heralding Jesus' birth.  Just putting myself in their shoes and imagining the craziness and absolute glory of that moment.

One of the best parts of it all was that Frazer and I got to go to an 11:00 Christmas Eve service.  We haven't done that in at least 6 years and honestly I wasn't sure I would be able to make it that late!  But I did and it was amazing to be there, worshipping and setting our hearts on things above as Christmas day began...a truly wonderful time for us both.

I promised myself to keep this relatively short so I'm going to sign off.  As always, thank you for your prayers (and everything else!) and we so appreciate your perseverance with us down this long road.  

Oh - and because so many of asked - My sister and her family are not moving to Memphis at this time.  They thought that is what God wanted them to do last summer but then God closed those doors, so they remain in Birmingham.  A dear friend told me recently, sometimes God just wants us to walk in obedience to Him, even if, in the end, it is not the ending that we had foreseen.  So for now, although we don't understand it and although it hurts our hearts, they are staying put.

Real quick, I have spoken of this before, but I want to say it again because it has stayed on my heart for weeks now and because it is such good truth...about Christmas and light and darkness…that Christmas means nothing if it doesn’t start with darkness.  Jesus was born in the dark.  He was resurrected in the dark.  God perhaps does His greatest work in the dark.  When the world couldn’t see its hand in front of its face, He showed up. 

(Sorry I'm still writing!  I get going and can't seem to stop!)

Ok.  We love y'all.  And goodnight.  And may the Lord keep us close to Himself no matter what 2016 holds...may we have joy and hope in abundance.

Dana

PS - Our Christmas card that didn't happen again this year :)